skip to main content

HLH — RACP Paediatrics MCQ

Instant feedback + full explanation. One question, done properly.

HardHaematology & OncologyHLHRACP Paediatrics

A 6-year-old presents with fever, pancytopenia, hepatosplenomegaly, markedly elevated ferritin (>10,000 mcg/L), hypertriglyceridaemia, and low fibrinogen. Bone marrow shows haemophagocytosis. What is the diagnosis?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: A — Haemophagocytic lymphohistiocytosis (HLH)

HLH presents with fever, cytopenias, hepatosplenomegaly, hyperferritinaemia (often >10,000), hypertriglyceridaemia, low fibrinogen, elevated soluble IL-2 receptor, and haemophagocytosis on biopsy. It can be primary (genetic, e.g. perforin mutations) or secondary (triggered by infection, malignancy, or autoimmune disease). Treatment includes dexamethasone, etoposide, and HSCT for primary HLH.

Reference: ANZCHOG – 2024 – Treatment Guidelines: HLH